The Severe Chronic NeutropeniaInternational Registry

Autoimmune Neutropenia

(Presence of neutrophil-specific antibodies)

In neutropenic children aged 6 months to 4 years other than congenital neutropenia, presence of neutrophil-specific antibodies can result in increased destruction of the body's own blood neutrophils. This so-called autoimmune neutropenia is the most common cause for neutropenia of this age group. Although these infants lack peripheral blood neutrophils, they usually do not suffer from severe bacterial infections.

In the serum of these patients granulocyte-specific antibodies are detectable by different immunological blood tests that can be performed in specialised laboratories. If antibodies against neutrophils are identified, the patients should be kept under medical care, but may not necessarily require treatment with antibiotics or G-CSF.

Depending on the frequency of infections and the neutrophil counts, prophylaxis with an oral antibiotic may be considered by the treating physician. In some children where severe infections occur, treatment with G-CSF is indicated. In most children the blood counts normalise during the first 2-3 years.

Autoimmune neutropenia is occasionally seen in young people (20 – 40 year age group) predominantly women and in this setting is often associated with other disorders or conditions.